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         Crigler-najjar Syndrome:     more detail
  1. Crigler-Najjar Syndrome

81. Crigler Najjar Syndrome (Bilirubin Uridinediphosphate Glucuronosylyltransferase
HOME Crigler Najjar syndrome (Bilirubin Uridinediphosphate GlucuronosylyltransferaseDeficiency). Type I Treatment of criglernajjar
http://www.bdid.com/crigler.htm

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Crigler Najjar Syndrome (Bilirubin Uridinediphosphate Glucuronosylyltransferase Deficiency)

82. ILDS: ICD-10 - By Code [E80200 - E88801]
This is a list of Dermatology disease codes that include Dercum's. Scan down to the bottom of the Category Health Conditions and Diseases Dercum Disease...... E80290 Porphyria (NOS), E805 syndrome criglernajjar, E805 crigler-najjarsyndrome, E83000 syndrome Menkes', E83000 Steely hair disease,
http://www.ilds.org/icd10/bycode/E-02.html
Application to Dermatology of ICD-10 Alphabetized by ICD Code, E80200 - E88801:
Return to Alphabetical listing...
ICD Code: Disorder Porphyria hepatic Porphyria Swedish Porphyria acute intermittent Hepatic porphyria Acute intermittent porphyria Swedish porphyria Porphyria variegate Variegate porphyria Porphyria South African genetic South African genetic porphyria Porphyria toxic Toxic porphyria Pyrrolopompholyx Chester porphyria Porphyria Chester Erythropoietic coproporphyria Hepatoerythropoietic porphyria Hereditary coproporphyria Hereditary protocoproporphyria Protocoproporphyria hereditary Coproporphyria hereditary Plumboporphyria Porphyria secondary Secondary porphyria Hematoporphyria (NOS) Porphyria (NOS) Syndrome Crigler-Najjar Crigler-Najjar syndrome Syndrome Menkes' Steely hair disease Disease steely hair Menkes' syndrome Disease Wilson's Wilson's disease Bronze diabetes Diabetes bronze Hemochromatosis Syndrome Troisier Hanot Chauffard Troisier Hanot Chauffard syndrome Acrodermatitis enteropathica Cystic fibrosis with skin manifestations Mucoviscidosis with skin manifestations Amyloidosis inherited systemic Familial Mediterranean fever Fever familial Mediterranean Muckle Wells syndrome Syndrome Muckle Wells Amyloidosis secondary systemic Amyloidosis primary localised cutaneous Amyloidosis secondary localised cutaneous Amyloidosis macular Macular amyloid Lichen amyloidosus Amyloidosus lichen Amyloidosis papular Papular amyloidosis Amyloidosis nodular Nodular amyloidosis Purpura amyloid

83. NORD - National Organization For Rare Disorders, Inc.
Crigler Najjar syndrome Type I. Copyright 1997, 1998, 2002 Synonyms of CriglerNajjar syndrome Type I Bilirubin Glucuronosyltransferase Deficiency Type I;
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Crigler Najja

84. Crigler Najjar Syndrome Type I
National Organization for Rare Disorders, Inc. Crigler Najjar syndrome Type I. CriglerNajjarsyndrome type I is inherited as an autosomal recessive trait.
http://www.bchealthguide.org/kbase/nord/nord1084.htm
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National Organization for Rare Disorders, Inc.
Crigler Najjar Syndrome Type I
Important
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Bilirubin Glucuronosyltransferase Deficiency Type I Uridine Diphosphate Glucuronosyltransferase, Severe Def. Type I Congenital Familial Nonhemolytic Jaundice Type I
Disorder Subdivisions
  • None
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
  • Crigler-Najjar Syndrome Type II Gilbert Syndrome Rotor Syndrome Rh Disease
General Discussion
Crigler-Najjar syndrome type I is a very rare inherited metabolic disorder characterized by the complete absence of the enzyme uridine diphosphate glucuronosyltransferase (UDPGT), which is normally found in the liver. This enzyme is required for the conversion (conjugation) and subsequent excretion of bilirubin from the body. When UDP-glucuronosyltransferase activity is absent, these metabolic processes are hampered and abnormally high levels of bilirubin accumulate in the blood (hyperbilirubinemia).
Within the first few days of life, most infants with Crigler-Najjar syndrome type I develop persistent yellowing of the skin, mucous membranes, and whites of the eyes (jaundice). In addition, some affected individuals may develop kernicterus, a potentially life-threatening neurological condition in which toxic levels of bilirubin accumulate in the brain, causing damage to the central nervous system. Early signs of kernicterus may include lack of energy (lethargy), vomiting, fever, and/or unsatisfactory feedings. More serious signs of kernicterus may develop, including abnormal muscle rigidity, resulting in muscle spasms (dystonia) and involuntary movements of the limbs and/or entire body (athetosis). Crigler-Najjar syndrome type I is inherited as an autosomal recessive genetic trait.

85. Reuters Health Information (2001-10-19): Crigler-Najjar Treated With Anesthetic-
FLORENCE, Italy (Reuters Health) A team of Italian physicians has treated Crigler-Najjarsyndrome type I with an infusion of donor liver cells in a procedure
http://www.gastro.org/reuters/2001/Oct/22/20011019clin019.html
Clinical Crigler-Najjar treated with anesthetic-free hepatocyte infusion Last Updated: 2001-10-19 9:31:58 EDT (Reuters Health) By Rossella Lorenzi FLORENCE, Italy (Reuters Health) - A team of Italian physicians has treated Crigler-Najjar syndrome type I with an infusion of donor liver cells in a procedure that required no anesthesia. Dr. Giovanni Ambrosino and colleagues announced this week that they had successfully completed the 3-hour operation at Padua's First Surgical Department on October 11th. The patient was a 10-year-old Croatian boy. Up to 16 hours of phototherapy daily is the mainstay of treatment for the condition, in which the liver does not produce bilirubin UDP-glucuronosyltransferase. Liver transplantation can be effective but the number of donor organs is limited and it requires life-long immune suppression. "Our goal is to avoid a whole liver transplant. Infusion of liver cells proved to be relatively easy to accomplish," said Dr. Ambrosino, director of the University of Padua's Bioartificial Liver Program. The infusion involved 7.5 ml of hepatic cells isolated from the left lobe of a donor liver. The cells were infused over a 3-hour period via catheter inserted 2 weeks previously under local anesthetic.

86. VADA GEZONDHEID En ZIEKTE - HEALTH And DISEASE A
SYNDROOM van CRIGLER NAJJAR CRIGLER NAJJAR syndrome. See also AANGEBOREN AFWIJKINGENSee also HYPERBILIRUBINEMIE FYSIOLOGISCHE GEELZUCHT HYPERBILIRUBINEMIA;
http://www.vada.nl/medisch/medcru.htm
VADA - GEZONDHEID en ZIEKTE
HEALTH and DISEASE
CV - CZ
OPGELET - ATTENTION
Patienten en leken die raadgevingen/adviezen/informatie zoeken via deze verzameling links wordt dringend geadviseerd de verzamelde informatie te bespreken met de (behandelend) arts/specialist/hulpverlener.
Patients and lay persons looking for guidance among the target sources of this collection of links are strongly advised to review the information retrieved with their professional health care provider.
CRACK
See also: DRUGSVERSLAVING DRUG ADDICTION
  • NRC: Soorten Drugs
  • Drugfree America Drug Info: Crack
    CRANIOFACIALE AFWIJKINGEN CRANIOFACIAL ANOMALIES CRANIOFACIAL DISORDERS CRANIOFACIAL GENETIC DISEASES
    CRANIOFACIAL GENETIC DISORDERS
    See also: ORBITA
    See also: CRANIOSYNOSTOSE CRANIOSYNOSTOSIS
    See also: SYNDROOM van APERT APERT SYNDROME
    See also: SYNDROOM van CARPENTER CARPENTER SYNDROME
    See also: SYNDROOM van COHEN COHEN SYNDROME
    See also: SYNDROOM van CROUZON CROUZON SYNDROME See also: SYNDROOM van MUENKE MUENKE's SYNDROME See also: See also: SYNDROOM van SAETHRE CHOTZEN SAETHRE CHOTZEN SYNDROME See also: SYNDROOM van TREACHER COLLINS TREACHER COLLINS SYNDROME TREACHER COLLINS-FRANCESCETTI SYNDROME MANDIBULOFACIAL DYSOSTOSIS See also: AANGEZICHTSSPLETEN TELEORBITISME See also: MICROTIE MICROTIA See also: PROGRESSIEVE HEMIFACIALE ATROFIE
  • CraniofaciaalCentrum AZR-Sophia
  • Friendly Faces: Craniofacial Disorders
    CRANIOFACIALE STENOSE
    See also: ORBITA See also: SYNDROOM van CROUZON CROUZON SYNDROME See also:
  • 87. Utah1999
    the Clinic for Special Children in Lancaster County, PA, where a group of Amish childrenwith a different metabolic disease, Crigler Najjar syndrome, will be
    http://members.aol.com/slo97/slonews9.html
    Smith-Lemli-Opitz Syndrome - Research News - Summer, 1999 The Utah Meeting Other Research News Richard Kelley, M.D., Ph.D.
    Kennedy Krieger Institute
    Johns Hopkins University September 15, 1999

    88. Liver Diseases
    Please Visit Our Liver Disease Pages listed below Liver Diseases Alagille SyndromeAlpha1-Antitrypsin Deficiency Autoimmune Hepatitis Biliary Atresia
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    Categories Alagille Syndrome Glycogen Storage Disease Alcoholic Liver Disease Hemochromatosis ...
    American Liver Foundation national, voluntary nonprofit health agency dedicated to preventing, treating, and curing hepatitis and all liver diseases through research, education, and support groups.
    Division of Digestive and Liver Diseases - Diseases of the Liver Resource provides an alphabetical list of liver diseases and conditions with links to more detailed information.
    American Association for the Study of Liver Diseases (AASLD) NIH Consensus Development Conference Management of Hepatitis C: 2002 Important Drug Alerts ....
    Dr. Wu's Liver Diseases

    89. WebGuest - Open Directory : Health : Conditions And Diseases : Genetic Disorders
    Top Health Conditions and Diseases Genetic Disorders CriglerNajjarSyndrome (5). See also Health Conditions and Diseases Nutrition
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    the entire directory only in Genetic_Disorders/Crigler-Najjar_Syndrome Top Health Conditions and Diseases Genetic Disorders : Crigler-Najjar Syndrome

    See also:
    Sites:
    • Austin Health Information - An overview of crigler-najjar syndrome including symptoms, treatment and prevention.
    • Crigler-Najjar Disease - Bi-lingual site with information and links on this disease.
    • Davidson College Biology Department - A brief description of crigler-najjar syndrome along with some links for further study.
    • NORD - General information about crigler najjar syndrome type I, followed by further resources.
    • Pediatric Database - A look at crigler-najjar syndrome, a definition, epidemiology, pathogenesis, clinical features, investigation and management.
    Last update: 4:53 PT, Wednesday, January 29, 2003
    Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor

    90. Bilirubin Disorders
    Disorders in Bilirubin Metabolism.
    http://www.indstate.edu/thcme/mwking/bilirubindisorders.html
    Disorders in Bilirubin Metabolism
    back to Inborn Errors Page Return to Medical Biochemistry Page Michael W. King, Ph.D / IU School of Medicine / mking@medicine.indstate.edu Last modified: Thursday, 10-May-01 10:11:17

    91. ÍغѵԡÒóì¢Í§âä Crigler-Najjar Type II And Gilbert's Syndrome
    The summary for this Bihari page contains characters that cannot be correctly displayed in this language/character set.
    http://www.thailiverclub.org/board1/00408.html
    Webboard Ëéͧ»Ö¡ÉÒá¾·Âì: Íغѵԡҳì¢Í§â¤ Crigler-Najjar type II and Gilbert's syndrome ã¹»Ðà·Èä·Â ¢ÍàÕ¹¶ÒÁà¡ÕèÂǡѺÍغѵԡҳì¢Í§â¤ Crigler-Najjar type II áÅÐ Gilbert's syndrome ã¹»Ðà·Èä·Â ÇèÒÁÕ¡ÒÒ§ҹäÇéË×ÍäÁè Ë×ͨÐá¹Ð¹ÓáËÅ觢éÍÁÙÅ㹡Ҥé¹ËÒ
    email :
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    ¡Ø³Ò¤ÅÔê¡ Post message à¾Õ§¤Ñé§à´ÕÂǤѺ....

    92. Listings Of The World Health Conditions And Diseases Genetic
    Austin Health Information Post Review An overview of criglernajjarsyndrome including symptoms, treatment and prevention. http//www
    http://listingsworld.com/Health/Conditions_and_Diseases/Genetic_Disorders/Crigle

    93. Auxiliary Liver Transplant
    The patient was a 10 1/2 month old infant who was born with CriglerNajjarsyndrome, a so-called inborn error of metabolism. This
    http://www.transweb.org/news/news/archive/aux_liver.html
    Auxiliary Liver Transplant at UMMC
    by Jeremiah G. Turcotte, MD
    Professor of Surgery, University of Michigan Organ Transplantation Center The first auxiliary liver transplant in Michigan was performed at UMMC in July, 1994. The patient was a 10 1/2 month old infant who was born with Crigler-Najjar syndrome, a so-called inborn error of metabolism. This disease results from an abnormality in the genes of all cells including those of the liver. The liver is unable to excrete the bile that accumulates in the blood from the natural breakdown of red blood cells. With Crigler-Najjar syndrome the liver cannot form bilirubin gluconate, a necessary step for bile to be transported from the blood stream to the bile ducts and then into the intestines. If not corrected within the first year of life, the high level of bile in the blood irreversibly damages the brain, a condition called kernicterus, and eventually leads to death. With an auxiliary liver transplant the patient's own liver remains in place and another whole or partial (reduced size) liver is transplanted just beneath or adjacent to the recipient's own liver. This is advantageous to patients with some types of inborn errors of metabolism, such as Crigler-Najjar syndrome, because the function of their own livers is perfectly normal except for the single genetic defect. With an auxiliary transplant the patient is not completely dependent upon the transplanted liver and should the auxiliary transplant reject or be lost for any reason, the patient's own liver will continue to function. Another auxiliary transplant can be performed under elective rather than emergent circumstances.

    94. Bilirubin Disorders
    Michael W. King, Ph.D / Medical Biochemistry / Terre Haute Center for Medical Education/memwk@thcme.indstate.edu Sergio MARCHESINI/Professor of Biochemistry
    http://www.med.unibs.it/~marchesi/bilirubindisorders.html
    Disorders in Bilirubin Metabolism
    Gilbert Syndrome

    Crigler-Najjar Syndrome Type I

    Crigler-Najjar Syndrome Type II

    Dubin-Johnson Syndrome
    ...
    Rotor Syndrome
    Michael W. King, Ph.D / Medical Biochemistry / Terre Haute Center for Medical Education/ memwk@thcme.indstate.edu
    Sergio MARCHESINI/Professor of Biochemistry/University of Brescia / marchesi@med.unibs.it
    Last Updated: 25.05.2001

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